Results 11 to 20 of about 10,624 (209)

Selective reduction of bis(monoacylglycero)phosphate ameliorates the storage burden in a THP-1 macrophage model of Gaucher disease

Journal of Lipid Research, 2013
Bis(monoacylglycero)phosphate (BMP) assists lysosomal function by facilitating interaction of hydrolases and activator proteins with sphingolipid substrates.
Leanne K. Hein, Stephen Duplock, Maria Fuller   +2 more
doaj   +1 more source

[29] Sphingolipid hydrolases and activator proteins

Biochimie, 2000
The physiological degradation of several membrane-bound glycosphingolipids (GSLs) by water-soluble lysosomal exohydrolases requires the assistance of sphingolipid activator proteins (SAPs). Four of these SAPs are synthesized from a single precursor protein (prosaposin).
Uwe Bierfreund, Thomas Kolter, Konrad Sandhoff   +2 more
  +7 more sources

Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion[S]

Journal of Lipid Research, 2010
We examined the effect of Niemann-Pick disease type 2 (NPC2) protein and some late endosomal lipids [sphingomyelin, ceramide and bis(monoacylglycero)phosphate (BMP)] on cholesterol transfer and membrane fusion.
Misbaudeen Abdul-Hammed, Bernadette Breiden, Matthew A. Adebayo, Jonathan O. Babalola, Günter Schwarzmann, Konrad Sandhoff   +5 more
doaj   +1 more source

Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases

Disease Models & Mechanisms, 2017
Sphingolipidoses are inherited diseases belonging to the class of lysosomal storage diseases (LSDs), which are characterized by the accumulation of indigestible material in the lysosome caused by specific defects in the lysosomal degradation machinery ...
Julia Sellin, Heike Schulze, Marie Paradis, Dominic Gosejacob, Cyrus Papan, Andrej Shevchenko, Olympia Ekaterina Psathaki, Achim Paululat, Melanie Thielisch, Konrad Sandhoff, Michael Hoch   +10 more
doaj   +1 more source

Degradation of blood group A glycolipid A-6-2 by normal and mutant human skin fibroblasts

Journal of Lipid Research, 1998
The degradation of blood group glycolipid A-6-2 (GalNAc(α1→3)[Fucα1→2]Gal(β1→4)GlcNAc(β1→3)Gal (β1→4)Glc(β1→1′)Cer, IV2-α-fucosyl-IV3-α-N-acetylgalactosaminylneolactotetraosylceramide), tritium-labeled in its ceramide moiety, was studied in situ, in skin
Befekadu Asfaw, Detlev Schindler, Jana Ledvinová, Bohuslav Černý, František Šmíd, Ernst Conzelmann   +5 more
doaj   +1 more source

A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

Neurobiology of Disease, 2017
Saposin deficiency is a childhood neurodegenerative lysosomal storage disorder (LSD) that can cause premature death within three months of life.
Samantha J. Hindle, Sarita Hebbar, Dominik Schwudke, Christopher J.H. Elliott, Sean T. Sweeney   +4 more
doaj   +1 more source

Next-Generation Sequencing Analysis Reveals Novel Pathogenic Variants in Four Chinese Siblings With Late-Infantile Neuronal Ceroid Lipofuscinosis

Frontiers in Genetics, 2019
Neuronal Ceroid Lipofuscinoses (NCLs) are progressive degenerative diseases mainly affect brain and retina. They are characterized by accumulation of autofluorescent storage material, mitochondrial ATPase subunit C, or sphingolipid activator proteins A ...
Xiao-Tun Ren, Xiao-Hui Wang, Chang-Hong Ding, Xiang Shen, Hao Zhang, Wei-Hua Zhang, Jiu-Wei Li, Chang-Hong Ren, Fang Fang   +8 more
doaj   +1 more source

Regulation of Mitogen-Activated Protein Kinases by Sphingolipid Products in Oligodendrocytes [PDF]

The Journal of Neuroscience, 1999
Sphingolipid products such as ceramide (cer), sphingosine (sph), and sphingosine-1-phosphate (SPP) are implicated in the regulation of cell growth and apoptosis. We have recently shown that cer, sph, and SPP differentially modulate ionic events in cultured oligodendrocytes (OLGs).
H, Hida, S, Nagano, M, Takeda, B, Soliven   +3 more
openaire   +2 more sources

Sphingolipid Activator Proteins Are Required for Epidermal Permeability Barrier Formation [PDF]

Journal of Biological Chemistry, 1999
The epidermal permeability barrier is maintained by extracellular lipid membranes within the interstices of the stratum corneum. Ceramides, the major components of these multilayered membranes, derive in large part from hydrolysis of glucosylceramides mediated by stratum corneum beta-glucocerebrosidase (beta-GlcCerase).
T, Doering, W M, Holleran, A, Potratz, G, Vielhaber, P M, Elias, K, Suzuki, K, Sandhoff   +6 more
openaire   +2 more sources

CPTP: A sphingolipid transfer protein that regulates autophagy and inflammasome activation [PDF]

Autophagy, 2018
The macroautophagy/autophagy and inflammasome pathways are linked through their roles in innate immunity and chronic inflammatory disease. Ceramide-1-phosphate (C1P) is a bioactive sphingolipid that regulates pro-inflammatory eicosanoid production. Whether C1P also regulates autophagy and inflammasome assembly/activation is not known. Here we show that
Shrawan Kumar Mishra, Yong-Guang Gao, Yibin Deng, Charles E. Chalfant, Edward H. Hinchcliffe, Rhoderick E. Brown   +5 more
openaire   +3 more sources